Long-term follow-up study after extracranial-intracranial bypass surgery for anterior circulation ischemia in childhood moyamoya disease

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✓ Between May, 1974, and March, 1991, 104 patients with moyamoya disease, all under 16 years old at the time of first surgery, underwent superficial temporal-to-middle cerebral artery anastomosis and/or encephalomyosynangiosis. The mean follow-up period was 9.6 years (range 4.8 to 16.0 years). Hemiplegia was the most frequent symptom before the first operation. Transient ischemic attacks (TIA's) were noted in 57 patients and minor stroke with hemiplegia in 44. The most frequent type of cortical dysfunction was aphasia (21 cases). Postoperatively, the incidence of TIA's and/or completed stroke with motor weakness of the extremities was markedly decreased, but visual disturbance progressed and major or minor stroke with visual disturbance was found in two cases. In patients under the age of 3 years, a major stroke prior to surgery resulted in a poor outcome in 36% of cases. Preoperative major stroke in patients between the ages of 3 and 7 years was less frequent, and poor outcomes were seen in 17% of this group. There were no major preoperative strokes in patients with surgery after the age of 7 years, and no poor outcomes were recorded in this group. A major preoperative stroke prior to surgery had adverse impact on the ultimate patient intelligence quotient (IQ) following surgery. All patients operated on after the age of 7 years had a normal or borderline IQ at follow-up examination.

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Address reprint requests to: Jun Karasawa, M.D., Department of Neurosurgery, Osaka Neurological Institute, 2–6–23 Shonai-Takara-machi, Toyonaka, Osaka 561, Japan.

© AANS, except where prohibited by US copyright law.

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    Illustrations showing surgical procedure. Left: The central branch of the middle cerebral artery (MCA) is dissected out after the dura mater is opened, and a piece of rubber is placed beneath a dissected portion. Right: The end of the superficial temporal artery is joined to the central branch of the MCA by an end-to-side anastomosis followed by encephalomyosynangiosis.

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    Angiograms in a patient with superfical temporal (STA)-to-middle cerebral artery (MCA) anastomosis and encephalomyosynangiosis. Left: Preoperative external carotid angiogram showing transdural anastomoses via the middle meningeal branch. Center: External carotid angiogram 3 months postoperatively. A major part of the MCA is filled via the STA and deep temporal artery. Right: Selective angiogram showing that a large part of the MCA is filled via the two arteries.

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    Graph showing the relationship between patient age at symptom onset and the clinical outcome as assessed by Glasgow Outcome Scale. Major stroke is found in 36.4% of patients under the age of 3 years, but no patients over 7 years of age had major stroke. Open circles denote transient ischemic attacks; closed circles denote reversible ischemic neurological deficits or minor stroke; triangles denote major stroke. Glasgow Outcome Scale grades: GR = good recovery; MD = moderate disability; SD = severe disability; PV = persistent vegetative state; and D = death (asterisk indicates one patient who died of primary pulmonary hypertension).

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    Graph showing the relationship between patient age at symptom onset and the postoperative intelligence quotient (IQ) as classified by Taft, et al.:13 N = normal; B = borderline; Mi = mild retardation; Mo = moderate retardation; S = severe retardation; and P = profound retardation. Open circles denote transient ischemic attacks; closed circles denote reversible ischemic neurological deficits or minor stroke; triangles denote major stroke. Asterisk = one patient who died of pulmonary hypertension. Eight patients (36.4%) with symptom onset earlier than 3 years of age had an IQ of less than borderline. All patients with onset of symptoms at over 7 years old had an IQ of either normal or borderline.

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    Graph showing the relationship between Glasgow Outcome Scale (GOS) score and the intelligence quotient (IQ). A statistically significant correlation is found between the two by the chi-squared test for independent variables (4 × 6 contingency table, χ2 160.807, p < 0.001). Open circles denote transient ischemic attacks; closed circles denote reversible ischemic neurological deficits or minor stroke; triangles denote major stroke. Asterisk = one patient who died of pulmonary hypertension. Grades of GOS: GR = good recovery; MD = moderate disability; SD = severe disability; PV = persistent vegetative state; D = death. Grades of IQ: N = normal; B = borderline; Mi = mild retardation; Mo = moderate retardation; S = severe retardation; P = profound retardation.

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    Graph showing the relationship between the operative results immediately after the last operation and Glasgow Outcome Scale (GOS) score. There is a significant correlation between the two (χ2 88.078, p < 0.001). Open circles denote transient ischemic attacks; closed circles denote reversible ischemic neurological deficits or minor stroke; triangles denote major stroke. Asterisk = one patient who died of pulmonary hypertension. Operative result classifications: CR = complete recovery; MI = marked improvement; SI = slight improvement; U = unchanged; W = worsened. Grades of GOS: GR = good recovery; MD = moderate disability; SD = severe disability; PV = persistent vegetative state; D = death.

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